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首页> 外文期刊>British Journal of Haematology >Disseminated lymphoblastic lymphoma in children and adolescents: Results of the COG A5971 trial: A report from the Children's Oncology Group
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Disseminated lymphoblastic lymphoma in children and adolescents: Results of the COG A5971 trial: A report from the Children's Oncology Group

机译：儿童和青少年的弥漫性淋巴母细胞淋巴瘤：COG A5971试验结果：儿童肿瘤学组的报告

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摘要

The Children's Oncology Group's A5971 trial examined central nervous system (CNS) prophylaxis and early intensification in paediatric patients diagnosed with CNS-negative Stage III and IV lymphoblastic lymphoma. Using a 2 × 2 factorial design, the study randomized patients to Children's Cancer Group (CCG) modified Berlin-Frankfurt-Muenster (BFM) acute lymphoblastic leukaemia (ALL) regimen with intensified intrathecal (IT) methotrexate (MTX) (Arm A1) or an adapted non-Hodgkin lymphoma/BFM-95 therapy with high dose MTX in interim maintenance but no IT-MTX in maintenance (Arm B1). Each cohort was randomized ± intensification (cyclophosphamide/anthracycline) (Arms A2/B2). For the 254 randomized patients, there was no difference in 5-year event-free survival (EFS) for the four arms: Arm A1, 80% [95% confidence interval (CI) 67-89%] and Arm A2, 81% (95% CI 69-89%); Arm B1, 80% (95% CI 68-88%) and Arm B2, 84% (95% CI 72-91%). The cumulative incidence of CNS relapse was 1·2%. Age <10 years and institutional imaging response at 2 weeks was associated with improved outcomes (P < 0·001 and P = 0·014 for overall survival). CNS positive patients (n = 12) did poorly [5-year EFS of 63% (95% CI 29-85%)]. For CNS-negative patients, there was no difference in outcome based on CNS prophylaxis (IT-MTX versus HD-MTX) or with intensification.
机译：儿童肿瘤小组的A5971试验检查了诊断为CNS阴性的III期和IV期淋巴母细胞性淋巴瘤的小儿患者的中枢神经系统（CNS）预防和早期强化。使用2×2因子设计，该研究将患者随机分为儿童癌症组（CCG）改良的柏林-法兰克福-明斯特（BFM）急性淋巴细胞白血病（ALL）方案和鞘内（IT）甲氨蝶呤（MTX）（Arm A1）或一种经调整的非霍奇金淋巴瘤/ BFM-95治疗，在中期维持期间使用大剂量MTX，但在维持期间不使用IT-MTX（手臂B1）。每个队列随机±强化（环磷酰胺/蒽环类）（A2 / B2组）。对于254位随机分组的患者，四组的5年无事件生存率（EFS）没有差异：A1组为80％[95％置信区间（CI）67-89％]，A2组为81％ （95％CI 69-89％）; B1组为80％（95％CI 68-88％）和B2组为84％（95％CI 72-91％）。 CNS复发的累积发生率为1·2％。年龄<10岁和2周的机构影像学反应与预后改善相关（总体生存率P <0·001和P = 0·014）。中枢神经系统阳性患者（n = 12）表现较差[5年EFS为63％（95％CI 29-85％）]。对于中枢神经系统阴性患者，基于中枢神经系统预防（IT-MTX与HD-MTX）或强化治疗的结局无差异。

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